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Home , Accessory spleen, Splenic infarction, Wandering spleen

Case Report World Journal of Surgery and Surgical Research Published: 25 Feb, 2020

Giant Accessory and Rotating Ectopic Kidney: A Rare Association

Diógenis Barbosa de Moura1, Lara Valeska de Medeiros Rocha1, João Luís Gomes da Silva1, Eusamar Magalhães Júnior1, Lauanda Ênia de Medeiros Rocha1, Antonio Bizerra Wanderley Neto1, Amália Cinhtia Meneses Rêgo2 and Irami Araújo-Filho3* 1Federal University of the State of Rio Grande do Norte, Brazil

2Department of Biotechnology, Potiguar University, Brazil

3Department of Surgery, Federal University of Rio Grande do Norte, Brazil

Abstract The is a congenital disability characterized by a separated ectopic splenic parenchyma. (WS) is a rare condition where the spleen, free from its ligaments, is allowed to move inside the abdomen, predisposing the patient to life-threatening complications due to torsion of the vascular pedicle; splenic , , and may occur. WS or Accessory Spleen (AS) is rarely suspected at presentation since symptoms are usually not specific, and definitive diagnosis is often reached only by imaging technologies such as color flow ultrasonography and angio-spiral computed tomography. The size is rarely more than 4 cm. Patients rarely present symptoms, and the diagnosis can be accidental when faced with ectopia in radiological or intraoperative investigations. It is an essential clinical entity since abdomen-pelvic tumors must always be considered as differential diagnoses, requiring detailed evaluation. Surgical treatment is indicated for symptomatic patients or those with complications. On the other hand, the ectopic kidney with rotation abnormality also consists of a singular entity. Congenital anomalies of OPEN ACCESS the upper urinary tract occur in 3% to 4% of those born, most of which are followed up clinically. *Correspondence: The present study discusses a case of symptomatic intrapelvic WS associated with an ectopic kidney Irami Araújo-Filho, Department of with rotation anomaly, a rare union, and still with scarce reports in the literature. This study aimed Surgery, Federal University of Rio to present the case of a patient with an isolated oversized accessory spleen and a review of the Grande do Norte, Av. Hermes da literature. Fonseca, 1444 - Apto. 1302 Tirol Natal Keywords: Spleen; Accessory spleen; Wandering spleen; Displaced spleen; ; State of Rio Grande do Norte, 59020- 650, Brazil, Tel: 5584988760206; Fax: 558433425079; Introduction E-mail: [email protected] The spleen is localized between the th9 and 11th left ribs in the abdominal cavity between the gastric Received Date: 25 Jan 2020 bottom and the left hemidiaphragm. With a weight of approximately 200 g, it represents the largest Accepted Date: 21 Feb 2020 lymphoid organ in the body [1-3]. It is fundamental for the hematological and immune system and Published Date: 25 Feb 2020 is an essential reserve of approximately 10% to 20% of the blood volume [4]. During its growth, the Citation: spleen can develop anomalies such as complete agenesis, multiple or , accessories de Moura DB, de Medeiros Rocha LV, spleens, and persistent lobulation. The Accessory Spleen (AS) is a congenital alteration resulting da Silva JLG, Júnior EM, de Medeiros from the failure to move the splenic tissue to its definitive position in the left hypochondrium. This is Rocha LÊ, Wanderley Neto AB, et al. a condition present in 10% of the population, located in regions such as splenic hilum or pancreatic Giant Accessory Spleen and Rotating tail, with an intrapelvic location being unusual [5,6]. Accessory Spleen (AS) may develop during Ectopic Kidney: A Rare Association. the sixth week of embryogenesis following the deposition of spleen cells along the path from the World J Surg Surgical Res. 2020; 3: midline, usually occurring on the left side. An AS is commonly located near the spleen’s hilum and 1202. in the tail, and is generally asymptomatic and diagnosed incidentally during laparotomy or Copyright © 2020 Irami Araújo- radiological examination performed for other reasons [7]. Adnexal masses typically originate from Filho. This is an open access article the genital tract; however, extrapelvic organs are rarely found in this location [8]. Although extremely distributed under the Creative rare, this possibility must be considered in the differential diagnosis of pelvic masses. Patients rarely Commons Attribution License, which present symptoms, and the diagnosis can be accidental when faced with ectopia in radiological or permits unrestricted use, distribution, intraoperative investigations [9-11]. It is an essential clinical entity since abdomen-pelvic tumors and reproduction in any medium, must always be considered as differential diagnoses, requiring detailed evaluation [12-14]. Surgical provided the original work is properly treatment is indicated for symptomatic patients or those with complications. On the other hand, the cited. ectopic kidney with rotation abnormality also consists of a singular entity. Congenital anomalies

Remedy Publications LLC., | http://surgeryresearchjournal.com 1 2020 | Volume 3 | Article 1202 Irami Araújo-Filho, et al., World Journal of Surgery and Surgical Research - General Surgery of the upper urinary tract occur in 3% to 4% of those born, most of which are followed up clinically [15-17]. The present study discusses a case of symptomatic intrapelvic AS associated with an ectopic kidney with rotation anomaly, a rare union, and still with scarce reports in the literature. Methods We searched the PubMed, Cochrane Database, Scopus, Web of Science, Embase, and Google Scholar databases (gray literature) using the keywords: Spleen, accessory spleen, wandering spleen; displaced spleen; abdominal pain; splenectomy. The selection of articles was made by two authors independently. A third author was Figure 2: CT scan of the showing an ectopic spleen with a vascular consulted in case of divergence in the inclusion or not of a particular pedicle. study. Reports related to accessory spleen were selected based on the chosen keywords, human studies, full-text articles, and published in English, Portuguese, or Spanish. Forty-three studies unrelated to the main objective of the review or outside the inclusion criteria were excluded after peer review. We included 26 articles from a total of 37 scientifically validated and relevant published studies. The Research Ethics Committee of Potiguar University - Natal/Brazil, approved the research after signature by the patient informed consent, according to Resolution 466/2012, of the National Commission of Ethics and Research in Humans - CONEP - Ministry of Health/Brazil. Protocol number 138/2019. Case Presentation A 32-year-old male, previously healthy, sought emergency care after opening a picture of sudden low back pain of extreme intensity, radiating to the testicles, in a burning character, with no history of previous pathology or abdominal trauma. He was medicated with Figure 3: 3D CT scan of the pelvis showing an ectopic pelvic spleen with a antispasmodics and , progressing with pain improvement. vascular pedicle and left kidney rotation in 180°. During evolution, an ultrasound examination was requested, revealing a single solid spherical nodule, with circumscribed margins, rectum and a left ectopic kidney rotating 180º in it longitudinal axis with similar, retrovesical hypoechogenic texture, with the central (Figure 2 and 3). After the exams, the patient developed arterial flow at Doppler (Figure 1), suggested further investigation by reporting ten days without fecal eliminations, without previous computed tomography. The tomographic examination confirmed the episodes of illness, taking him to the gastroenterology service, renal ectopia on the left and the presence of a solid mass similar to the where he was examined and submitted to a colonoscopy, which splenic parenchyma, showing a relationship with the posterior wall did not reveal any changes. He was treated with symptomatic and of the bladder and with the left seminal vesicle, an apparent cleavage laxative phytotherapeutics, improving and oriented to seek general plane with both, but with no evident cleavage plane with the upper surgery service. The patient was referred and evaluated for a surgical procedure to remove the mass found in the imaging exams. With no comorbidities, no family history of , or malformations, he underwent surgery to resolve a pilonidal cyst in childhood. We opted for exploratory laparotomy, with an encapsulated mass with a smooth violet-shiny appearance, measuring about 4.0 cm × 4.0 cm × 4.5 cm (Figure 4), with an ascending vascular pedicle invading the omentum bigger. The mass was resected and sent for anatomopathological study, which characterized tissue compatible with the accessory spleen, congested parenchyma, and atrophy of white pulp (Figure 5). After the procedure, the patient evolved without complications and was discharged in twenty-four hours, being reassessed fifteen days after the surgical intervention, favorable recovery, and no episode of pain in this period. Discussion The spleen is an encapsulated organ composed of lymphatic and vascular tissue, located in the left hypochondrium [8]. Some variations Figure 1: Pelvic ultrasound showing a single solid spherical nodule with in its development have already been observed, including complete circumscribed margins, with similar, retrovesical splenic hypoechogenic agenesis, polysplenia, isolated accessory spleens, and persistent texture, with the central arterial flow at doppler. lobulations [9,18]. The organ begins its evolution during the fifth

Remedy Publications LLC., | http://surgeryresearchjournal.com 2 2020 | Volume 3 | Article 1202 Irami Araújo-Filho, et al., World Journal of Surgery and Surgical Research - General Surgery

Figure 4: Exploratory laparotomy, with an encapsulated mass with a smooth violet-shiny appearance, measuring about 4.0 cm × 4.0 cm × 4.5 cm, with an ascending vascular pedicle invading the omentum bigger (Ectopic spleen with a vascular pedicle). week of embryonic life, from the condensation of mesenchymal especially in emergencies [17-19,24]. CT scans show a well-margined cells present between the layers of the dorsal mesogastrium [7,19]. mass, similar to the splenic parenchyma on the contrast phase. The Accessory Spleen (AS) results from the formation of ectopic Magnetic imaging can also be used to evaluate tissue aspects and splenic tissue or from failure to migrate the organ, from its original the vascular pedicle of the accessory spleen. Only nuclear medicine site, in the midline, to its final position, in the upper left abdomen. imaging can confirm the diagnosis with scintigraphy performed with An accessory spleen is defined as ectopic splenic tissue that develops 99mTc-labelled colloids or Tc-99 m heat-damaged red blood cells due to the failure of cell fusion during embryonic development while because the colloid labeled with Tc-99m is taken from the reticulum- migrating from the midline to the left upper quadrant [15,16]. They endothelium and makes visible the spleen, liver and bone marrow; can be localized commonly next to the hilus and vascular pedicle, the however, it is necessary to suspect the diagnosis of accessory spleen tail of the pancreas, left ovary or left testis, in the , for this procedure [22]. Therefore, often only surgical excision can and the of the small and the large intestine, along the safely confirm the diagnosis [23,25]. For these reasons, many surgical greater curvature of the and in the pouch of Douglas. procedures have been done for diagnosis. Laparotomy was performed Macroscopically, a typical accessory spleen usually appears as a solid for resection of the lesion and anatomopathological study, whose mass, 1 cm to 2.5 cm in diameter. Masses larger than 4 cm are very report confirmed splenic tissue. The open road was chosen due to the rare [2,5] with a smooth, round, ovoid, or minimally lobulated shape. retrovesical location and the team's more significant experience with Microscopically, it reproduces a splenic pattern. An accessory spleen this technique [14-16]. However, the laparoscopic approach is also commonly has a well-defined fibrotic capsule that separates the an option for treatment. Torsion, spontaneous rupture, hemorrhage, surrounding healthy tissue [18]. According to necropsy studies, AS and cystic formations are responsible for the clinical condition of is present in 10% of the population [20]. It’s most common locations symptomatic patients, but they are rare complications [9,18]. The are the splenic hilum (75%) and the tail of the pancreas (20%), as congestion demonstrated by light microscopy was the probable cause well as the greater omentum, greater curvature of the stomach, and of the symptoms in the patient in question. It is worth mentioning mesentery [1-3]. Unver Dogan et al. [1] investigated 720 autopsies, another anatomical variety presented by the patient, detected by and BA was found in 6.7% of cases, 2 of which were pelvic. The imaging exams (USG and CT): Ectopia and 180º rotation of the left patient in the present study presented intrapelvic AS, representing kidney, characterizing an exaggerated kidney over-rotation [11,17]. an atypical anatomical variety and with few reports in the scientific Congenital anomalies of the upper urinary tract involving kidneys literature. In addition to the congenital origin, in rare cases, its source and ureters occur in 3% to 4% of those born, with abnormalities in the is acquired, resulting from the autotransplantation of splenic tissue shape and position of the organs being the most common [7,23]. Most during splenectomy or after abdominal trauma, a process defined of these renal anomalies are followed clinically, requiring a correct as . However, because this patient does not have a history diagnosis of the morphological alteration, as well as the evaluation of of trauma or splenectomy, it is a congenital intrapelvic AS [21]. possible complications [24-26]. During the review of the literature on WS appears as an incidental finding, without clinical significance, the topic, the authors not found reports of an association between an with no preference for sex or race, asymptomatic and accidentally accessory spleen and an ectopic kidney. Despite this, this variety did diagnosed during radiological investigations or abdominal surgeries not affect the patient's symptoms in this case. [13-15]. Thus, the report of low back pain with irradiation to testicles Conclusion is another peculiarity of the present case, since symptoms, as a rule, are uncommon. Due to the clinical presentation, renal lithiasis In conclusion, the present study demonstrated a rare case of the was suspected, drugs were prescribed, and an abdominal pelvic accessory spleen associated with a 180° renal rotation that ultrasound was requested for diagnostic confirmation [18,22]. USG was only definitively elucidated in the intraoperative period. An did not show signs of nephrolithiasis but found a solid pelvic nodule accessory spleen can be variously located, and the pelvic position is with unspecific echographic characteristics. Rectal tumor, bladder hugely uncommon. Preoperative diagnosis is still tricky, especially in tumor, lymphadenomegaly, , retroperitoneal fibrosis, and an emergency, and as in our case, the literature shows the difficulty other masses of intrapelvic location form hypotheses suggested in these of reaching a diagnosis before surgery. The main misdiagnosis is a circumstances [14,23]. A CT scan was performed, given the demand neoplastic disease, and for this reason, accessory spleen could be for more accurate imaging evaluation. The report showed a nodule wrongly removed. For this reason, undiagnosed pre or intraoperative with behavior similar to the splenic parenchyma during the dynamic pelvic mass, firmly to the spleen, has to be managed carefully. Despite contrast phase when BA was then included in the list of differential pelvic AS being a rare, usually asymptomatic, pelvic mass condition, diagnoses. Preoperative diagnosis of the accessory spleen is difficult, it should be considered in the differential diagnosis of symptomatic

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